Surgical treatment of congenital kyphosis

STUDY DESIGN: In this study, 26 cases of congenital kyphosis and kyphoscoliosis treated surgically were retrospectively reviewed. OBJECTIVE: To assess the clinical outcomes and surgical indications for posterior only versus anteroposterior surgery in the child. SUMMARY OF BACKGROUND DATA: Congenital kyphosis usually is progressive without surgical intervention. Current recommended treatment includes posterior arthrodesis for deformities of less than 50 degrees to 60 degrees, and anterior release or decompression, anterior fusion, and posterior instrumented arthrodesis for large deformities and cord compression. METHODS: Cases involving myelodysplasia, spinal dysgenesis, and skeletal dysplasia were excluded from the study. Kyphoscoliosis was included if the kyphotic deformity was greater than the scoliotic deformity. Patients were grouped by age and surgical technique. The patients in group P1 underwent posterior arthrodesis at an age younger than 3 years, and those in group P2 underwent the procedure at an age older than 3 years. The patients in group AP1 underwent anterior and posterior procedures at an age younger than 3 years, and those in group AP2 underwent the procedures at an age older than 3 years. The preoperative deformity, complications, and postoperative deformity correction were analyzed. There were nine Type 1 (failure of formation), nine Type 2 (failure of segmentation), and eight Type 3 (mixed) deformities. Four patients had associated spinal dysraphism. Three patients with Type 1 deformities had clinical or radiographic evidence of cord compression. RESULTS: In Group P1, five patients at an average age of 16 months underwent posterior arthrodesis alone for an average kyphotic deformity of 49 degrees. The immediate postoperative correction improved over a period of 6 years and 9 months by an additional 10 degrees, resulting in a final deformity of 26 degrees. Pseudarthrosis developed in two patients, requiring fusion mass augmentation or anterior arthrodesis. Neither patient was instrumented. In Group P2, five patients at an average age of 13 years and 7 months underwent posterior arthrodesis with instrumentation for kyphotic deformity of 59 degrees. Approximately 30 degrees of intraoperative correction was achieved safely using compression instrumentation and positioning. No further correction occurred with growth. The final residual kyphotic deformity was 29 degrees after a follow-up period of 4 years and 5 months. In Group AP1, seven patients underwent anterior release or vertebra resection for deformity correction and posterior arthrodesis for an average kyphotic deformity of 48 degrees at the age of 16 months. There were no iatrogenic neurologic injuries. The final residual kyphotic deformity was 22 degrees after a follow-up period of 6 years and 3 months. In Group AP2, nine patients underwent anterior release or decompression with posterior arthrodesis for kyphotic deformity of 77 degrees at the age of 11 years and 6 months. The deformity was corrected to 37 degrees, with no significant loss over a follow-up period of 5 years and 2 months. There were two postoperative neurologic complications. CONCLUSIONS: After reviewing their experience, the authors made the following observations: 1) The pseudarthrosis rate was low even without routine augmentation of fusion mass if instrumentation was used; 2) gradual correction of kyphosis may occur with growth in patients younger than 3 years with Types 2 and 3 deformities after posterior fusion, but appears to be unpredictable; 3) the risk of neurologic injury with anterior and posterior fusion for kyphotic deformity was associated with greater age, more severe deformity, and preexisting spinal cord compromise.     

Natural history of congenital kyphosis and kyphoscoliosis. A study of one hundred and twelve patients.

BACKGROUND: Congenital kyphosis and kyphoscoliosis are much less common than congenital scoliosis. However, they are potentially more serious because compression of the spinal cord and paraplegia sometimes develop. The goals of the present study were to document the natural history of congenital kyphosis and kyphoscoliosis and to determine the stage at which the natural progression should be interrupted by treatment. METHODS: We reviewed the medical records and radiographs of the spine of 112 consecutive patients. Sixty-eight patients had a type-I kyphosis due to an anterior failure of vertebral-body formation, twenty-four had a type-II kyphosis due to an anterior failure of vertebral-body segmentation, and twelve had a type-III kyphosis due to a combination of anomalies; the deformities of the remaining eight patients could not be classified. Eighty-five skeletally immature, untreated patients were first evaluated at a mean age of six years and nine months (range, two months to sixteen years and three months), and twenty-seven patients were skeletally mature at the time of the first visit. Sixty-three of the eighty-five skeletally immature patients were observed without any treatment for a mean period of six years and six months (range, one to sixteen years) before skeletal maturity, and the remaining twenty-two patients had a posterior arthrodesis of the spine soon after the initial visit. At skeletal maturity, forty-one patients had not been treated and sixty-eight had had an arthrodesis of the spine. The remaining three patients had not yet reached skeletal maturity at the time of the most recent follow-up. RESULTS: The apex of the kyphosis was seen at all levels but was most frequent between the tenth thoracic and the first lumbar level (seventy-four patients; 66 percent). There was no relationship between the severity of the kyphosis and its location in the spine. Progression of the curve was most rapid during the adolescent growth spurt and stopped only at skeletal maturity. Progression was most rapid and the magnitude of the curve was the greatest in type-III kyphosis (twelve patients) followed by type-I kyphosis due to a posterolateral quadrant vertebra (thirty-nine patients), a posterior hemivertebra (eight patients), a butterfly vertebra (fifteen patients), and a wedged vertebra (six patients). A kyphosis due to two adjacent type-I vertebral anomalies progressed more rapidly and produced a more severe deformity than did a similar single anomaly. The prognosis for type-II kyphosis was variable and was much more severe when an anterolateral unsegmented bar had produced a kyphoscoliosis (nine patients) than it was when a midline anterior bar had produced a pure kyphosis (fifteen patients), which usually progressed slowly. Spontaneous neurological deterioration due to compression of the spinal cord occurred in ten patients (seven of whom had a type-I kyphosis and three of whom had an unclassifiable anomaly) at a mean age of thirteen years and eight months, and one other patient (with an unclassifiable anomaly) had spastic paraparesis at the age of twenty-eight years. CONCLUSIONS: Congenital kyphosis and kyphoscoliosis are uncommon deformities with the potential to progress rapidly, resulting in severe deformity and possible neurological deficits. A thorough knowledge of the natural history is essential in the planning of appropriate and timely treatment to prevent progression of the deformity and neurological complications.     

Simultaneous anterior-posterior approach through a costotransversectomy for the treatment of congenital kyphosis and acquired kyphoscoliotic deformities

BACKGROUND: Congenital kyphosis and acquired kyphoscoliotic deformities are uncommon but are potentially serious because of the risk of progressive deformity and possible paraplegia with growth. Our current approach for the treatment of these deformities is to use a single posterior incision and costotransversectomy to provide access for simultaneous anterior and posterior resection of a hemivertebra or spinal osteotomy, followed by anterior and/or posterior instrumentation and arthrodesis. To our knowledge, this approach has not been reported previously. METHODS: The medical records and radiographs for sixteen patients who had been managed at our institution for the treatment of congenital kyphosis and acquired kyphoscoliosis between 1988 and 2002 were analyzed. The mean age at the time of surgery was twelve years. The diagnosis was congenital kyphosis for fourteen patients and acquired kyphoscoliotic deformities following failed previous surgery for two. The mean preoperative kyphotic deformity was 65 degrees (range, 25 degrees to 160 degrees ), and the mean scoliotic deformity was 47 degrees (range, 7 degrees to 160 degrees ). Fifteen patients were managed with vertebral resection or osteotomy through a single posterior approach and costotransversectomy, anterior and posterior arthrodesis, and posterior segmental spinal instrumentation. The other patient was too small for spinal instrumentation at the time of vertebral resection. A simplified outcome score was created to evaluate the results. RESULTS: The mean duration of follow-up was 60.1 months. The mean correction of the major kyphotic deformity was 31 degrees (range, 0 degrees to 82 degrees ), and the mean correction of the major scoliotic deformity was 25 degrees (range, 0 degrees to 68 degrees ). Complications occurred in four patients; the complications included failure of posterior fixation requiring revision (one patient), lower extremity dysesthesias (one patient), and late progressive pelvic obliquity caudad to the fusion (two patients). The outcome, which was determined with use of a simplified outcomes score on the basis of patient satisfaction, was rated as satisfactory for thirteen patients, fair for two patients, and poor for one patient. CONCLUSIONS: A simultaneous anterior and posterior approach through a costotransversectomy is a challenging but safe, versatile, and effective approach for the treatment of complex kyphotic deformities of the thoracic spine, and it minimizes the risk of neurologic injury. LEVEL OF EVIDENCE: Therapeutic Level IV.     

Thoracic kyphoscoliosis resembling neurofibromatosis: a case report focusing on subfascial instrumentation.

BACKGROUND CONTEXT: The management of early-onset progressive scoliosis is controversial. PURPOSE: To describe the unusual surgical management of a young female with an early-onset progressive, short, angular kyphoscoliosis resembling neurofibromatosis. STUDY DESIGN: A case report reviewing the treatment of an unusual occurrence of kyphoscoliosis. METHODS: After compliant Milwaukee brace wear had failed to stop deformity progression, surgical management including segmental anterior and posterior T5-T10 arthrodesis, and posterior T3-L4 subfascial rod instrumentation was performed at age 4 years and 6 months. After 10 rod lengthenings, segmental anterior T10 to L3 arthrodesis and posterior T3-L4 instrumentation and arthrodesis were done at age 11 years and 5 months. RESULTS: From preoperative to 3-year postoperative definitive surgery, her T3 to L4 instrumented spine length increased by 9.5 cm; 4 cm after the initial surgery, 3 cm between the initial surgery and the definitive surgery and 2.5 cm after the definitive surgery. From preoperative to latest follow-up, her thoracic scoliosis was reduced from 89 to 31 degrees, her thoracolumbar compensatory scoliosis from 59 to 37 degrees and her kyphosis from 70 to 17 degrees. CONCLUSIONS: The combination of early definitive anterior and posterior major curve arthrodesis with a subfascial rod lengthening program to control the remainder of the thoracolumbar spine was a satisfactory solution for this unusual case of early-onset, progressive, short, angular kyphoscoliosis.     

Double-segment total vertebrectomy for the surgical treatment of congenital kyphoscoliosis: a case report

BACKGROUND CONTEXT: Congenital kyphosis or kyphoscoliosis is an uncommon deformity that usually is progressive without surgical intervention. In the lately diagnosed or neglected cases of congenital kyphoscoliosis, the patients may come with shoulder-trunk imbalance anomalies, severe deformity in coronal and sagittal plane, rib cage deformities, pelvic tilt, presence of intramedullary anomalies, neurological deficit, and difficulty in walking and cardiopulmonary problems. PURPOSE: To present a technical note related with double-segment total vertebrectomy for the surgical treatment of a patient who had neglected congenital kyphoscoliosis in lumbar spine. STUDY DESIGN: Case report. METHODS: A 19-year-old girl had submitted to our center with complaints of deformity and pain in her back. Her physical examination revealed scoliosis and gibbosity in lumbar region. Her neurological examination was normal. In the radiological examination, X-ray films showed 42 degrees lumbar scoliosis in frontal plane and 35 degrees kyphotic curvature in the sagittal plane. RESULTS: Three-staged (posterior-anterior-posterior) surgery in the same session (same anesthesia) was performed. CONCLUSION: Total or partial vertebrectomy on the apex of the deformity and the adjacent vertebral bodies along with anterior stabilization by means of a cylindrical cage combined in one operative procedure preceded by temporary posterior instrumentation and followed by posterior instrumentation and fusion may be preferred for the treatment of congenital kyphoscoliosis in neglected cases to provide spinal cord decompression.     

Surgical treatment of Scheuermann's disease with segmental compression instrumentation

Twenty-one patients with Scheuermann's kyphosis had surgery for progressive kyphotic deformity of 50 degrees or greater. There were six adolescents, with a mean age of 15.6 years (range, 13-17 years) and 15 young adults, with a mean age of 25.4 years (range, 18-40 years). All patients had posterior spine arthrodesis with segmental compression instrumentation. Seven patients with rigid kyphosis had combined anterior and posterior spine arthrodesis. One patient died of superior mesenteric artery syndrome. In the group of 13 patients with posterior arthrodesis only, followup was 4.5 years. The mean preoperative thoracic kyphotic curve of 68.5 degrees improved to 40 degrees at latest review, with an average loss of correction of 5.75 degrees. Junctional kyphosis occurred in two patients with a short arthrodesis: one at the cephalad end and one at the caudal end of the fused kyphotic curve. In the second group of seven patients with combined anterior and posterior arthrodesis, followup was 6 years. The mean preoperative thoracic kyphotic curve of 86.3 degrees improved to 46.4 degrees at latest review, with an average loss of correction of 4.4 degrees. Overall, there was no postoperative neurologic deficit and no pseudarthrosis. Thus, posterior arthrodesis and segmental compression instrumentation seems to be effective for correcting and stabilizing kyphotic deformity in Scheuermann's disease. Despite a long operating time, this technique provided significant correction, avoiding the development of any secondary deformity in most patients. Combined anterior and posterior spine arthrodesis is recommended for rigid, more severe kyphotic deformities.     

Pitfalls of spinal deformities associated with neurofibromatosis in children

A study of 116 patients younger than 12 years of age conclusively diagnosed as having neurofibromatosis was undertaken to determine the incidence of significant orthopedic problems. Deformities of the spine comprised the most common skeletal problem. Seventy-four patients (64%) had spinal deformities. Forty-six patients were treated by posterior spinal fusion. Ten required exploration for pseudarthrosis; six were found to have pseudarthrotic defects in the fusion mass. Eight patients had more kyphosis than scoliosis. Only three patients with kyphoscoliosis obtained a solid posterior spinal fusion after multiple surgical procedures. Anteroposterior and lateral roentgenograms of the cervical spine are recommended at the time of initial evaluation of all spinal deformities. Four patients had severe cervical spine deformities, only one of whom was initially identified as having a cervical spine disorder while under treatment for scoliosis. Three of these patients were seen by other surgical services for neck masses. Following removal of posterior elements, the osseous structures were unstable. Only one patient developed spondylolisthesis. Because of the exceedingly high incidence of pseudarthrosis and spinal instability following attempts at spinal fusion, certain guidelines have evolved for the management of these deformities. High-volume computed tomographic myelography in the prone, lateral, and supine positions or magnetic resonance imaging should be performed on all patients prior to surgical treatment. Anterior disc excision and bone graft followed by posterior arthrodesis with instrumentation are indicated if the kyphotic angle is greater than 50 degrees or if scoliosis is greater than 80 degrees. Even combined anterior and posterior arthrodesis operations did not guarantee successful permanent spinal stability in young patients with neurofibromatosis.     

一期后路经椎弓根半椎体切除节段固定治疗先天性胸腰椎侧后凸畸形

目的：评价一期后路经椎弓根半椎体切除节段固定术治疗完全分节的胸腰椎半椎体畸形所致的先天性脊柱侧后凸畸形的疗效。方法：采用一期后路经椎弓根半椎体切除内固定矫形术治疗了12例完全分节的胸腰椎半椎体畸形所致的脊柱侧后凸畸形，其中，男8例，女4例，年龄7～17岁，平均11．3岁。观察并测量术前术后及随访时站立位脊柱X线片冠状面和矢状面Cobb角、侧凸的顶椎偏移。结果：随访10-34个月，平均16个月。术后冠状面平均矫正64．1％，随访中无明显丢失。后凸由术前平均320矫正至190，随访中无明显丢失，顶椎偏移由术前4．5cm矫正至1．2cm。无脊髓损伤及切口感染等并发症。结论：一期后路经椎弓根半椎体切除内固定矫形术是治疗完全分节的胸腰椎半椎体畸形所致的脊柱侧后凸畸形的有效方法。     

Posterior Vertebral Column Resection for VATER/VACTERL Associated Spinal Deformity: A Case Report

The VATER/VACTERL association is a syndrome notable for congenital vertebral malformations, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, and renal or limb malformations. Vertebral malformations may include the entire spectrum of congenital spinal deformities, including kyphosis, as was seen in this case. A 14-year-old girl presented to our institution with severe rigid sagittal deformity in the thoracolumbar spine that had recurred following three prior spinal fusion surgeries: the first posterior only, the second anterior and posterior, and the third a posterior only proximal extension. These surgeries were performed to control progressive kyphosis from a complex failure of segmentation that resulted in a 66° kyphosis from T11 to L3 by the time she was 9 years old. Our evaluation revealed solid arthrodesis from the most recent procedures with resultant sagittal imbalance, and surgical options to restore balance included anterior and posterior revision spinal fusion with osteotomies, multiple posterior extension osteotomies with circumferential spine fusion, and posterior vertebral column resection with circumferential spine fusion. She was advised that multiple posterior extension osteotomies would likely be insufficient to restore sagittal balance in the setting of solid arthrodesis from anterior and posterior surgery, and that the posterior-only vertebral column resection would provide results equivalent to revision anterior and posterior surgery, without the morbidity of the anterior approach. She successfully underwent posterior vertebrectomy and circumferential spinal fusion with instrumentation and is doing well 2 years postoperatively. Severe rigid sagittal deformity can be effectively managed with a posterior-only surgical approach, vertebrectomy, and circumferential spinal fusion with instrumentation. An erratum to this article can be found at     

Orthopaedic manifestations of familial dysautonomia. A review of one hundred and thirty-six patients

BACKGROUND: Familial dysautonomia is a hereditary multisystemic disease primarily affecting people of Ashkenazi Jewish descent. Musculoskeletal problems are related to gait disorders, spinal deformities, foot deformities, fractures, and arthropathies. METHODS: The charts and radiographs of 136 patients who ranged in age from three months to forty-six years (mean, sixteen years) were reviewed. Sixty-four patients were available for follow-up examination. RESULTS: Spinal deformity was the most common orthopaedic problem and was diagnosed in seventy-eight patients starting at the age of four years, with a prevalence of 86 percent (forty-eight of fifty-six) by the age of fifteen years. Forty-one (53 percent) of the seventy-eight patients had scoliosis only, thirty-four (44 percent) had kyphoscoliosis, and three (4 percent) had kyphosis only. Bracing was accompanied by emotional, pulmonary, and skin problems, leading to a high rate of noncompliance and progression of the curve. Twenty-four patients had an operation at a mean age of thirteen years (range, five to eighteen years): twenty patients had posterior spinal arthrodesis, and four had combined anterior and posterior arthrodesis. Fifteen patients had a total of nineteen complications, of which seven were systemic and twelve were related to the spinal fixation. Eight patients had revision surgery. At the time of the surgery, scoliosis was corrected from a mean of 55 degrees to a mean of 35 degrees and kyphosis was corrected from a mean of 69 degrees to a mean of 61 degrees. After a mean duration of follow-up of sixty-five months, scoliosis measured 49 degrees (range, 18 to 62 degrees) and kyphosis measured 67 degrees (range, 30 to 115 degrees). Postoperative progression of the deformity was caused by failure of the instrumentation or progression in unfused segments. Walking was delayed in 72 percent (ninety-four) of the 130 patients who were of walking age. All sixty-four of the patients who were examined had an ataxic gait. Foot deformities were found in sixteen patients, six of whom were treated surgically. Two patients had Charcot joints. Fifty-five patients sustained at least one fracture before skeletal maturity, with a mean of 1.5 fractures per patient. All but one of the fractures was treated nonoperatively, and fracture-healing was often accompanied by profuse callus formation. CONCLUSIONS: Spinal deformity is common in patients with familial dysautonomia. Bracing is of questionable benefit, and surgical intervention should be considered once curve progression is well documented. Arthrodesis should be extended as far proximally as possible to prevent junctional kyphosis. Swelling and warmth in a limb should raise suspicion of an undiagnosed fracture.     

胸腰段陈旧骨折继发后凸畸形的外科治疗

目的总结分析胸腰段陈旧骨折继发后凸畸形的手术治疗效果。方法回顾研究胸腰段陈旧骨折继发后凸畸形33例,平均年龄40 3岁。病史平均36 0个月。后凸Cobb角平均40 8°(20°~82°)。全部患者均有脊髓损伤,括约肌功能障碍26例。12例有显著腰背部疼痛。23例既往曾有手术史。手术方式包括前路椎体间隙松解植骨+后路截骨矫形固定15例,后路截骨减压矫形12例,前路松解、椎体间撑开植骨固定6例。结果全部患者后凸畸形矫正率平均为86 0%。无严重手术并发症。随访时间平均24 6个月(6个月~84个月), 32例骨性融合, 1例植骨未融合,行二次手术后骨性融合。10例术后神经功能有改善, 10例术后括约肌功能部分恢复。有显著腰背部疼痛者术后症状均明显减轻。结论单纯后路截骨矫形适用于角度较小( <45°)的后凸畸形。前路松解、后方经关节突截骨矫形适用于不同程度的后凸畸形,尤其对后凸严重或二次手术的病例更显优势。对于合并脊髓不全损伤的病例即使病史较长,手术仍然可能获得一定疗效,特别是对于腰部疼痛的缓解效果显著。     

Posterior vertebrectomy in kyphosis, scoliosis and kyphoscoliosis due to hemivertebra

Vertebrectomy and instrumentation only via the posterior approach has been increasingly used in sagittal, frontal plane and combined deformities. The aim of this retrospective study is to evaluate the clinical and radiological results of hemivertebrectomy and instrumentation only via the posterior approach in congenital spinal deformities. Between 1998 and 2003, we performed hemivertebrectomy and interbody fusion using posterior instrumentation with titanium mesh cage via the posterior approach in 19 patients (three scoliosis, five kyphosis and 11 kyphoscoliosis). The age of the patients ranged from 2 to 22 years and they all underwent hemivertebrectomy (at thoracic level in six patients, at thoracolumbar level in eight patients and at lumbar level in five patients). A titanium mesh cage was used for anterior column support and interbody fusion in patients who had residual anterior gap preventing bone-to-bone contact. Correction and stabilization were achieved by posterior polyaxial pedicle screws. Follow-up was an average of 4.6 years (range: 2-7 years). We did not confront any loss of correction, pseudoarthrosis, and titanium mesh cage collapse or implant failure. Hemivertebrectomy and instrumentation via the posterior approach is a good one-stage surgical treatment option that can be used to avoid the surgical trauma and morbidity related to anterior surgery. It is a technically demanding surgical procedure, however, requiring extreme care and experience in spine surgery.     

Surgical correction of kyphosis: posterior total wedge resection osteotomy in 32 patients

BACKGROUND: Many surgical procedures have been developed for the treatment of kyphoscoliosis. We present our experience of one technique for posterior total wedge resection osteotomy, with clinical and radiographic results. This is a one-stage solution which results in a shortened posterior column and reduced tension on the spinal cord in rigid angular kyphosis. PATIENTS AND METHODS: Between 1990 and 2000, we treated 32 patients with rigid local kyphosis by posterior wedge osteotomy and instrumentation. The etiology was congenital malformation in 17 cases, infection in 11 and previous laminectomy in 4 cases. The osteotomy is performed at the apex of the kyphotic deformity and covers two vertebrae. The upper and lower borders of the osteotomy are right inferior to the transverse processes of the upper and lower vertebrae respectively. The apex of the posteriorly based triangular osteotomy is either at the anterior vertebral body or anterior longitudinal ligament. RESULTS: The mean preoperative angle of local kyphosis was 72 (25-112) degrees mainly at the thoracolumbar region, and it improved to a mean of 23 (0-48) degrees after an average follow-up of 57 (24-108) months. The mean preoperative sagittal plumbline imbalance of 5.5 (2-12) cm was improved to 1.2 (-2-3.5) cm postoperatively. The mean loss of correction since operation was 3.4 (0-11) degrees. Radiographically, solid anterior and posterior fusion was achieved in all patients by 6 months. 1 patient had irreversible paraplegia and 2 others had transient nerve root injury postoperatively. INTERPRETATION: Posterior total wedge resection osteotomy eliminates the need for anterior procedure and does not cause tractional force on the spinal cord, since the posterior column is shortened. This is an effective one-stage procedure, especially for the treatment of sharp and rigid kyphosis.     

Neurofibromatosis type I with severe dystrophic kyphoscoliosis and its operative management via a simultaneous anterior-posterior approach: a case report and review of the literature.

BACKGROUND CONTEXT: Neurofibromatosis is an autosomal-dominant hereditary disorder with two subtypes: NF-1 (type I) and NF-2 (type II). NF-1 is a complex disorder with a constellation of manifestations that can also entail skeletal abnormalities, including spinal deformity of a noncongenital nature with early age onset. The short, sharp, angular curve usually present in the thoracic region, as exhibited in NF-1, presents a quandary in its surgical management. Various studies have reported on the efficacy of anterior correction as opposed to posterior alone, whereas others have advocated a sequential, combined approach to diminish the degree of deformity and achieve solid arthrodesis. However, despite solid arthrodesis, curve progression may still ensue. Nonetheless, a simultaneous anterior-posterior approach to treat such a condition of NF-1 with severe dystrophic kyphoscoliosis is a rare occurrence. PURPOSE: To describe the presentation and operative management of a patient with NF-1 and severe dystrophic kyphoscoliosis. STUDY DESIGN: A case report and review of the literature. METHODS: A clinical and radiographic review of a 51-year-old male patient who presented with NF-1, a 165-degree thoracic kyphotic deformity, associated scoliosis, varied degree of vertebral destruction of T9-T11, and paraparesis below T10. RESULTS: Operative intervention of the deformity consisted of a simultaneous anterior-posterior approach and entailed posterior cord exposure, anterior vertebrectomy of T9-T11, cord decompression, posterior osteotomy (posterior elements were auto-fused), anterior distraction and kyphosis correction, anterior strut grafting, anterior rod instrumentation, and posterior compression instrumentation and fusion from T6-L2. The deformity was reduced, sold fusion was noted, and the patient was asymptomatic. CONCLUSIONS: A simultaneous anterior-posterior approach for the surgical treatment of severe dystrophic kyphoscoliosis in neurofibromatosis type I is an avenue to properly visualize the spinal cord, achieve solid arthrodesis, and to minimize as well as prevent the progression of deformity.     

Pseudarthrosis in adult spinal deformity following multisegmental instrumentation and arthrodesis

BACKGROUND: There have been few detailed reports concerning pseudarthrosis following spinal instrumentation and arthrodesis in adults with spinal deformity since the introduction of modern segmental fixation techniques. The purposes of this study were to analyze the prevalence, risk factors, and outcome scores on the Scoliosis Research Society Instrument-24 associated with pseudarthrosis following instrumentation and arthrodesis for the treatment of spinal deformity in adults. METHODS: A clinical and radiographic assessment of 232 adults with spinal deformity who were treated surgically at a single institution was conducted. The average age of the patients was 40.8 years, and the operation was a primary procedure in 150 patients and a revision procedure in eighty-two patients. All patients who underwent a long (four vertebrae or more) spinal instrumentation and arthrodesis with a minimum follow-up of two years were included in the analysis. Clinical outcomes were assessed with the Scoliosis Research Society questionnaire. RESULTS: Forty patients had a pseudarthrosis. Factors that were found to be significantly associated with pseudarthrosis were preoperative thoracolumbar kyphosis of >20 degrees (p < 0.0001), an age of more than fifty-five years (p = 0.001), arthrodesis to S1 compared with arthrodesis to L5 or a cephalad level (p = 0.002), and arthrodesis of more than twelve vertebrae (p = 0.037). Patients with a pseudarthrosis had lower total outcome scores on the Scoliosis Research Society questionnaire, on the average, than those without a pseudarthrosis (p = 0.001). CONCLUSIONS: The prevalence of pseudarthrosis following long arthrodesis with use of modern segmental spinal instrumentation for the treatment of spinal deformity in adults was 17%, and the clinical outcome in these patients can be negatively affected by the pseudarthrosis.     

Lung function in congenital kyphosis and kyphoscoliosis

OBJECTIVE: To quantify the respiratory compromise in patients with a congenital kyphosis or kyphoscoliosis in whom the major deformity is the kyphosis. METHODS: Forty-one patients with congenital vertebral anomalies resulting in a kyphosis or kyphoscoliosis, in which the kyphosis was the major deformity, and requiring spine surgery were studied retrospectively. The preoperative respiratory function and radiographic spinal assessments were compared. RESULTS: Twenty-two patients (54%) had an impairment of respiratory function: 2 patients (5%) were severely affected, 8 patients (20%) were moderately affected, and 12 patients (29%) were mildly affected. CONCLUSIONS: An increasing severity of kyphosis was associated with a significant increase in respiratory impairment (P<0.005). A more cranial level of the kyphosis, especially above T10, had a significantly greater effect on respiratory impairment (P<0.001). One untreated patient with a severe kyphosis (128 degrees) died from cor pulmonale.     

一期后路病灶清除、楔形截骨矫形治疗胸腰椎结核并后凸或侧后凸畸形

目的探讨一期后路病灶清除、楔形截骨矫形及内固定治疗胸、腰椎结核并后凸或侧后凸畸形的可行性、应用指征及临床意义。方法对16例胸、腰椎结核并后凸或侧后凸畸形的患者采取一期后路病灶清除、楔形截骨矫形及内固定术。男性12例,女性4例,年龄17~53岁,平均37 1岁。术前后凸角为54°~138°,平均78 3°; 2例合并侧凸,角度分别为31°和24°; 1例伴随侧方移位。7例合并脊髓损害, 2例为C级, 5例为D级, 2例表现为马尾及神经根损害。结果手术时间为215~325min,平均265min,术中出血450~2200ml,平均1100ml。术后结果满意, 14例为优, 2例为良,无严重并发症发生,脊髓神经功能损害均获显著改善。术后后凸角度为0°~67°,平均28 5°,矫正率63 6%,侧凸和侧方移位基本矫正。随访14 ~52个月,平均26 3个月。无内固定松动、断裂及明显矫正度丢失,融合良好。结论对胸、腰椎结核并后凸或侧后凸畸形的患者采取一期后路病灶清除、楔形截骨矫形及内固定术是一较为有效、可行的方法。与联合前、后路手术比较,该技术减少了创伤且有更好的畸形矫正效果。     

Closing-opening wedge osteotomy to correct angular kyphotic deformity by a single posterior approach

STUDY DESIGN: Seven patients with angular kyphotic deformity of the thoracic or thoracolumbar spine were treated by closing-opening wedge osteotomy using a single posterior approach. OBJECTIVES: To examine the safety and efficacy of closing-opening wedge osteotomy for angular kyphosis. SUMMARY OF BACKGROUND DATA: Correction osteotomy of severe kyphosis is a challenging operation. A two-stage operation has been commonly used: anterior release and decompression followed by posterior correction and fusion. METHODS: Seven patients with angular kyphosis were treated. The apex level of kyphosis was T5 in one patient, T11 in one, and T12 in five. There was old fracture in five patients, congenital deformity in one, and neurofibromatosis in one. The first 30-35 degrees of kyphosis are corrected using the closing wedge technique with the hinge of the anterior longitudinal ligament after veretebrectomy and circumspinal decompression of the spinal cord. Then the hinge is moved posteriorly to the spinal cord and the remainder of the requisit angle of osteotomy is corrected using the opening-wedge technique (closing-opening wedge osteotomy). Spinal curvature is stabilized using posterior instrumentation and graft. RESULTS: Localized kyphosis was reduced from an average of 67 degrees to 18 degrees at 2.2 to 7.5 years' follow-up. Sagittal alignment from T1 to the sacrum became more physiologic than before. There were no neurologic complications. Bony fusion was achieved in all patients, and there was no correction loss. CONCLUSIONS: Satisfactory correction is safely performed by closing-opening wedge osteotomy with a direct visualization of the circumferentially decompressed spinal cord. Although the performance is technically laborious, it offers good correction without jeopardizing the integrity of the spinal cord.     

后路全脊椎截骨术治疗胸腰段陈旧骨折继发后凸畸形

目的总结全脊柱截骨矫正胸腰段陈旧性骨折继发后凸畸形的治疗经验。方法陈旧性骨折继发脊柱后凸畸形17例,采用全脊柱截骨、椎弓根钉内固定系统固定并牵引闭合截骨间隙,植骨融合。结果术后全组病例均未发生脊髓损伤,临床症状得到不同程度的改善。17例平均随访18个月（8个月～6年）,术后所有病例截骨间隙均达到骨性融合,无一例发生假关节、内固定松动。结论经全脊柱截骨视野开阔,操作安全方便,截骨和内固定同时完成,是治疗脊柱后凸畸形的较好方法。     

The role of posterior instrumentation and fusion after anterior radical debridement and fusion in the surgical treatment of spinal tuberculosis: experience of 127 cases

Long periods of immobilization, progressive kyphosis and graft failure are the major postoperative problems encountered after anterior radical surgical treatment for tuberculosis of the spine. Posterior fusion and instrumentation can be an effective solution for these problems. Effectiveness of posterior fusion and instrumentation was investigated in this study on the basis of the cases with anterior procedure only, and with combined anterior-posterior procedures. One hundred twenty-seven cases of tuberculosis of the spine were surgically treated between 1987 and 1995. All had either 1 or more of conditions such as spinal cord compression and neurological deficit, vertebral body collapse and kyphosis, or wide paravertebral abscess unresponsive to medical treatment. Of these, 57 had only anterior radical procedure between the years 1987 and 1993. Seventy cases had posterior instrumentation and fusion after the anterior procedure between the years 1991 and 1995. In about two third of the patients (81) autogenous iliac strut graft and in one third of them (40) autogenous fibular strut graft (cases with more than 2 level involvement) was used along with rib grafts after debridement. Twenty-one of the 57 patients who had only anterior procedure demonstrated a postoperative increase of kyphosis of more than 10 degrees. Increased kyphosis was due to graft slippage in 3, resorption in 2 and subsidence in 16 patients. No such increase or graft failure was noted in cases of combined anterior-posterior procedure. The difference in terms of kyphosis was found to be statistically significant (P=0.047). Anterior radical debridement and strut graft is the golden standard in the surgical treatment of spinal tuberculosis, but it should always be accompanied by posterior instrumentation and fusion to shorten the immobilization period and hospital stay, obtain good and long lasting correction of kyphosis, and prevent further collapse and graft failure.